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Congenital Nystagmus Treatment & Management

  • Author: Mark Ventocilla, OD, FAAO; Chief Editor: Edsel Ing, MD, FRCSC  more...
 
Updated: Mar 24, 2016
 

Medical Care

The treatment of these patients should begin with correction of any refractive error. Amblyopia should be addressed as well.

Contact lens wear has been noted to diminish infantile nystagmus, presumably by a trigeminal efferent pathway. This may also increase foveation time by avoiding induced spectacle distortion with ocular movement in patients with high degrees of ametropia.

Refractive errors, even low plus, should be prescribed, as this has been shown to improve visual acuity. An optical system with high plus spectacles and high minus contact lenses has also been shown to improve visual acuity in some patients.

Base-out prisms (eg,7 prism diopters) may be combined with -1.00 D myopic overcorrection for convergence damping in patients with binocular vision.[5]

Pharmacologically useful agents for patients with nystagmus are primarily GABA agonists or inhibitors of the excitatory neurotransmitter system. The only drug found to be of benefit in adult patients with a history of idiopathic infantile nystagmus is baclofen. This drug has not been approved for use in children. Baclofen has been effective in treating the periodic alternating nystagmus (PAN) subtype.

Recent case reports have shown gabapentin to be beneficial in congenital nystagmus, with an improvement in foveation time and vision and a decrease in amplitude and frequency of the nystagmus.

McLean et al investigated treatment of congenital nystagmus with memantine or gabapentin in a randomized, double-blind, placebo-controlled trial of 48 patients.[6] Improvement in mean visual acuity was noted in both treatment groups (F=6.2; p=0.004, analysis of variance). Patients with afferent visual defects showed poorer improvements in visual acuity to medication than those with apparently normal visual systems. However, nystagmus intensity (F=7.7; p=0.001) and foveation (F=8.7; p=0.0007) improved in both nystagmus forms as shown on eye movement recordings. Patients in both treatment groups reported vision improvement more often than patients in the placebo group (p=0.03). McLean et al concluded that memantine and gabapentin can improve visual acuity, reduce nystagmus intensity, and improve foveation in congenital nystagmus.

More study is needed to determine the role of oral and topical carbonic anhydrase inhibitors.[5]

Alternative measures, such as biofeedback, acupuncture, or cutaneous head and neck stimulation, have been reported to decrease nystagmus in select (adult) patients with a history of infantile nystagmus.

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Surgical Care

Retrobulbar or intramuscular injection of botulinum toxin

Retrobulbar or intramuscular injection of botulinum toxin (BOTOX®) has been demonstrated to abolish nystagmus temporarily, but patient satisfaction has been poor due to adverse effects, such as ptosis or diplopia, and the need for reinjection.

Strabismus surgery

Strabismus surgery is used in patients with certain forms of nystagmus with varying degrees of success.

Anderson or Kestenbaum procedures are used to move the eyes into the null zone to diminish an anomalous head position in the setting of idiopathic infantile nystagmus.

Recession or simple tenotomy of all 4 horizontal rectus muscles has been advocated; however, preliminary results have been mixed. A pilot study has been completed that showed some improvement, but the definitive study is still pending.[7]

Surgery occasionally is used in the treatment of superior oblique myokymia.

Hertle et al conducted a prospective interventional case study to determine the effects of early eye muscle surgery on 19 patients younger than 24 months who had infantile nystagmus syndrome.[8] Outcome measures included acuity, head position, strabismic deviation, and eye movement recordings, including waveform types and a nystagmus optimal foveation fraction (NOFF). Improvements were noted in all outcomes measured. Hertle et al concluded that early eye muscle surgery in patients with oculographically infantile nystagmus syndrome improves the oscillation and visual functions. Increases in foveation periods and the NOFF were measureable.

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Consultations

Pediatric neurology consultation can be helpful in patients suspected of harboring CNS disease and in evaluating nystagmus for localizing a CNS lesion.

Pediatric endocrinology consultation may be useful in patients with optic nerve hypoplasia to assist in evaluation of pituitary function.

Pediatric metabolic disease specialists can offer assistance in patients with congenital cataracts or optic atrophy who are thought to have an underlying metabolic abnormality.

Pediatric geneticists play an increasing role in the diagnosis and management of patients with nystagmus whose diagnosis is uncertain or who have dysmorphic features.

Pediatric neuroradiologists are of considerable value in evaluating the infant brain for abnormalities uncovered with neuroimaging.

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Contributor Information and Disclosures
Author

Mark Ventocilla, OD, FAAO Adjunct Clinical Professor, Michigan College of Optometry; Editor, American Optometric Association Ocular Surface Society Newsletter; Chief Executive Officer, Elder Eye Care Group, PLC; Chief Executive Officer, Mark Ventocilla, OD, Inc; President, California Eye Wear, Oakwood Optical

Mark Ventocilla, OD, FAAO is a member of the following medical societies: American Academy of Optometry, American Optometric Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, Southern Medical Association, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Chief Editor

Edsel Ing, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Hospital for Sick Children and Sunnybrook Hospital

Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Royal College of Physicians and Surgeons of Canada, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, Canadian Society of Oculoplastic Surgery, European Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Medical Association, Ontario Medical Association, Statistical Society of Canada, Chinese Canadian Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

Michael J Bartiss, OD, MD Medical Director, Ophthalmology, Family Eye Care of the Carolinas and Surgery Center of Pinehurst

Michael J Bartiss, OD, MD is a member of the following medical societies: American Academy of Ophthalmology, North Carolina Medical Society, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Acknowledgements

Theodore Curtis, MD Assistant Professor, Department of Ophthalmology, University of Colorado; Consulting Staff, Rocky Mountain Lions Eye Institute

Theodore Curtis, MD is a member of the following medical societies: American Academy of Ophthalmology and American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

David T Wheeler, MD Associate Professor, Departments of Ophthalmology and Pediatrics, Oregon Health & Science University

David T Wheeler, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and Phi Beta Kappa

Disclosure: Nothing to disclose.

References
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  17. Lennerstrand G, Nordbo OA, Tian S, Eriksson-Derouet B, Ali T. Treatment of strabismus and nystagmus with botulinum toxin type A. An evaluation of effects and complications. Acta Ophthalmol Scand. 1998 Feb. 76(1):27-7. [Medline].

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